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Panchakarma A Ray of Hope for Duchenne Muscular Dystrophy

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Received 20/08/19 Accepted 24/10/19 Published 10/11/19

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Int J Ayu Pharm Chem REVIEW ARTICLE www.ijapc.com

e-ISSN 2350-0204

ABSTRACT

The Duchene muscular dystrophy is a genetic disorder. It affects every 1: 3600 live male births

due to mutation in dystrophin gene. DMD is characterized by progressive symmetrical

muscular weakness that affects proximal muscles more than distal. Patient lose the ability to

stand, walk and loss of ambulation before 10 years, with progression of the disease most

patients succumb to death in their early 20s.In Ayurveda, it has been classified under

Adibalapravrittavyadhi. Here pathogenesis occur due to the Beejabhagavayava dushti which

lead to Medomamsa dushti further vitiates the Vata.So Vata as the prime dosha to cause

neurological disorders, vitiates rasa, rakta, mamsa, jala, agni & oja and leads to gradual

progression of muscle wasting.In Ayurvedic Rasayana group medicines and specified

Panchakarma therapies are more effective and helpful on Dhatu Kshaya, which enhance

quality of the life and longer survival upon Duchene muscular dystrophy.

KEYWORDS

DMD, Dystrophin , Adibalapravruttavyadhi, Medomamsa Dusti , Panchakarma

Panchakarma A Ray of Hope for Duchenne Muscular Dystrophy

Lalita Sharma1*, Alok Srivastava2, Parul Sharma3, Priya Kutiyal4, Bhuvnesh Sharma5 and

K.K. Sharma6

1-6Department of Panchakarma, U.A.U, Rishikul Campus Haridwar, India

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INTRODUCTION

The word dystrophy derived from Latin and

Greek roots meaning of faulty nutrition.

The disease was first described by

Neapolian physician Giovanni Semmola in

18341. Dystrophinopathies are a group of

disorders resulting from mutations in the

dystrophin gene Duchenne muscular

dystrophy (DMD) & Becker muscular

dystrophy (BMD) combinedly known as

DBMD are the most succeeding

dystrophies to be suffered by children all

over the globe. Prevalence of DMD is three

times higher than BMD. DMD is one of the

serious form of recessive X – linked

inherited disorder primarily affecting

skeletal and cardiac muscles2. Dystrophin is

cytoskeletal protein and it forms

dystroglycal-glycoprotein complex. This

complex maintains the integrity of muscle

cells. Absence of dystrophin protein results

in the destabilization of the entire

dystroglycan-glycoprotein complex, hence

muscle mass does not grow well which

leads to weakness of muscles. Mostly

symptoms appear in the skeletal muscles,

Cardiac and Phrenic Muscles. Most patients

die because of heart failure or respiratory

problems. Each child of a carrier mother

has a 50% chance of inheritance of DMD.

Diagnosis of these disorders is based on

clinical presentation, genetic testing,

Muscle biopsy and muscle imaging. No any

treatment is at present in DMD definitely in

any medical field. In Modern

corticosteroids, physical therapy,

respiration assistance and gene therapy is

administered to the patient. With the

increasing prevalence of disease in young

children, multiple centers pertaining to

special care are also foundered in India3.

After birth, the Sequencing of presentation

starts with mild delayed milestones during

toddler period, often toe – walking,

difficulty to raise from floor and frequent

falls. By the age of 5 years a marked

disparity between physical ability and

surrounding peers occur. During 2nd decade

of life; respiratory, cardiac & orthopaedic

involvement takes place and without any

medical intervention, leads to a cost of life

expectancy (at the age of 18 to 20 years)4.

In Ayurveda, the disease cannot be directly

correlated to any of a particular disease

entity. This disease can be attributed under

the concept of adibalapravritta vyadhi5

Khavaigunya of mamsavahastrotas causing

dhatvagni impairment6. DMD can be

considered as an imbalance of vatadosha,

saptadhatu (basic elements for formation of

garbha both as functional & structural - to

the level of dhatwagni) and ojas

considering its progressive degeneration to

systemic involvement. The cardinal feature

is chestahani (decreased mobility), which

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indicates decrease in chalaguna. Recent

advancements are addressing towards

multi-systemic complications. Various

treatment advances are been undertaken,

currently FDA has accepted EXONDYS

51™ (eteplirsen) injection7 . Which is

useful for DMD children with a confirmed

deletion / mutation of Exon 51 only8. Stem

cell therapy and gene therapy are still in the

preliminary stages of development.

Absence of specific treatment makes it

more important to consider complementary

and alternate approach of management.

According to Ayurveda, for nourishment,

strengthening and rejuvenation of

mamsadhatu we should administer Balya

(strenthening), Rasayana (rejuvenative) ,

Agnivardhana (digestive & carminative) &

Vatadoshahara drugs . Panchakarma

therapy in the form of Abhyanga,

Shalishashtika Pindasweda, Basti has been

taken along with Ayurvedic regimen render

the patient longer survival with muscular

dystrophy.

AIMS AND OBJECTIVES

1) To study etio-pathogenesis of DMD

through both modern and Ayurveda

perspectives.

2) To promote Ayurvedic treatment

principles in managing DMD.

MATERIALS AND METHODS

This review was done by compiling the

classical Ayurveda literature, Ayurveda

Paediatric books, modern paediatric books,

magazines, research journals, thesis and

dissertations Pub med, WHO guidelines for

Muscular Dystrophy, AIIMS guidelines,

CCRAS database, websites etc.

Confirmation of the diagnosis

1. Serum Creatine kinase 9

2. Routine blood Investigation

3. Muscle biopsy

4. The genetic tests commonly used to

identify dystrophin mutations are multiplex

PCR10 .

5. Multiplex ligation-dependent probe

amplification11.

6. Single-condition amplification/internal

primer12.

7. Multiplex amplifiable probe

hybridization13.

CLINICAL MANIFESTATION

The clinical features of muscular dystrophy

depends on the age group of child for

example- In infancy: In this age group boys

are rarely symptomatic at birth or in early

infancy, although some are mildly

hypotonic.

Insidious onset with gradually progressive

course.

Positive Gower’s sign14.

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Difficulty in climbing upstairs and getting

up from sitting position, pelvic muscles

involvement more than shoulder muscles.

Pseudohypertrophy in some muscle and

atrophy in others.

Cardiomyopathy: Persistent tachycardia

and myocardial failure, is affected in 50-

80% of patients with DMD.

Intellectual impairment: 20-30% has an

IQ <70 in case of DMD affected children’s.

The learning disabilities are mostly found

in affected patients but still allow them to

function in a regular classroom, particularly

with remedial help.

PATHOGENESIS

Pathogenesis can be given by the concept of

Adibala Parvritta Vyadhi viz., Sushruta's

vyadhi vargikarana 15 and let it Asadhya

Roga16.

DIAGNOSTIC CRITERIA

Patients presenting with the clinical picture

of proximal muscle weakness, difficulty

while standing, climbing staircase, pseudo-

hypertrophy of calf muscles, positive

Gower’s sign, 6min walking test with

confirmative test as serum CPK values,

testing for DMD mutation in blood sample

(Genetic test - multiplex PCR), EMG –

NCV Test, Histopathological report.

DISSCUSION

Ayurveda and DMD:

According to Acharya charak20 , the very

definite cause for defective progeny is

vitiation of beeja & beejabhaga. The part in

beejabhaga is vitiated that anga originating

from the part of beeja will be deformed and

this can be related to genetic diseases due to

chromosomal disorders. So, this can be

related to the cause of disease effecting X

chromosomes and thus causing DMD in

male child and females as carrier.

Role of vata, rasa, rakta, mamsadhatu,

jala, agni and ojas in DMD

Mamsa dhatu is formed by conjoining of

rasa , rakta with vata , jala & agni21.

Acharya vagbhata says , vata is the main

cause for birth of deformed child. When it

gets vitiated, it dries up the channels of rasa

etc dhatu (lack of nourishment to foetus)

leading to newborn suffering from further

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vatavyadhi (neurological disorder) or born

with birth defects. These both can be

considered as the reason for dystrophy in

children as a genetic origin24 . During

Dhatu nirmana (tissue formation) , katu

awasthapaka gets vitiated due to

derangement in agni thus afflicting Vata ,

produces visham mahabhuta ; which turn

into improper formation of dhatu. Further,

Dhatu parmanu is also produced

abnormally because of these visham

mahabhuta and now the destruction occurs

by swabhava (natural). This finally depletes

the oja causing respiratory & cardiac

complications22.

Samprapti:

The pathogenesis of dystrophy can be

understood by Mamsavaha srotodushti

vikara which occur due to defect in shukra

/ shonita (deformity in X chromosome) or

vitiated matruja bhavas (sudden mutation

of gene) leading to beejabhaga /

avayavadushti. Due to beejadushti,

vatavaishamyata (disproportionate) occurs,

causing improper formation of mamsa

dhatu by the influence of dhatwagni of

rakta & mamsa23. Due to decrease in

dhatwagni there is formation of ama

(indigestion) and due to this faulty nutrition

it causes progressive degeneration of

mamsadhatu (muscle tissue)24.While

srotorodha (obstruction due to metabolic

waste) produces hypertrophy of particular

region and so first occurs as prokopa and

then depletion of vata element takes place.

Roopa:

The clinical features can be correlated as

Mamsa kshaya of sphik (muscle wasting of

thigh), mamsasphik, uru, jangha vrudhi

(hypertrophy of muscles of thigh, chest,

abdomen and hip), gurugatrata (heaviness

of muscles) adhimamsa (hyperplasia of

muscle), prabhrutayo mamsadoshaja

(inflammation of necrosis of muscle) 25.

Chikitsa:

The basic line of management concentrates

on correction of dhatuparinama prakriya.

Ayurveda considers the significance of

agni, which is the main factor for

uttarottaradhatu (every next tissue)

formation. Thus correction of this

dhatwagni, by administering deepana and

pachana drugs strengthen the dhatu and

further elimination of metabolic waste is to

be done26. Acharya vagbhata said that the

usage of rukshana dravya for bruhmhana

treatment modalities; as a pre-operative

procedure which helps in removal of

srotorodha and do sthirikarana of anga27

.Though vata is the prime dosha to

neurological conditions and basti is

considered as ardha chikitsa and ultimate

amongst all but still in conditions like

DMD, a multi-dimensional approach

should be followed.

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The Proposed line of treatment for DMD

First line –Srotosodhana

Dhatwagni deepana-pacana

(rukshana)

Second line – Dhatukshaya janya

Vatavyadhi Chikitsa (to promote tissue

metabolism)

Third line –Brumhana chikitsa.

PANCHAKARMA IN DMD

1. Deepana and Pachana (like udvartana,

pariseka with dhanyamla – (at tissue level)

2. Snehapana28 – with Tikta Gruta,

Amritprasha Gruta, Indukantam Ghruta,

3. SarvangaAbyanga with

Balaashwagadha-lakshadi Taila,

Mahanarayana Taila, Mahamashadi

Taila29,

4. Swedana (Shastikashali pinda sweda30,

patrapotlipinda sweda31, mamsakizhi)

5. Virechana with Trivtrut Leha (best in

children).

6. Basti (of brumhana property) - Mamsa

rasa basti, Mustadi-yapana basti

7. Anuvasana basti with Ashwagandha

Ghruta, Changalyadi Ghruta32

8. Nasya with Masha Taila, Kshirabala

Taila

Mechanism of action of Panchakarma on

DMD

In India, with this incidence and no cure in

contemporary system of medicine.In

Ayurveda, Paraspar dhatu paka concept is

of prime importance33. Dhatu paka avastha

clearly signifies the importance of Agni

which is responsible for the formation of

the next dhatus. In Panchakarma, Deepana

and pachana dravyas are given to correct

agni , balanced doshas and for elimination

of metabolic toxins from the Dhatu34. In

Poorva karma Acharyas gave the concept

of "Brhmanyastu mrudu langyet " in which

for better Brihmana treatment Rukshana

modalities are used 35 . Udvartana helps in

Sthiri karana of angas and the removal of

srotorodha. Initially for Deepana

parishekha with Dhanyamla Dhara can be

done 36-37 Panchakarma procedures i.e.

Vamana, Virechana, Niruha, Anuvansana

and Nasya are of prime importance38.

Vamana of mrudu kind i.e. if the person is

present with kapha sthana gata pitta or

utkilshta kapha lakhashanas, then using the

drugs like Madana phala which has

anapaitava as guna, has least

complications and it corrects the depleted

Medas39-40. Vacha as dravya for the vamana

can be given because it gives better

improvement in children for the

neuromuscular disorder 41. Mrudu

Virechana is explained under Vatsya

upkarma and it has anulomana as well as

tridoshahara property42. Amritprasha

ghruta and Tikta ghrutas are used as

shodhana snehapana, because tikta rasa is

Srotoshodhaka. Virechana does the

detoxification which lead to better

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absorption of Rasyana Drugs, other

Brihmana Dravyas and correction of Agni

43. In Gambhir Dhatu gata vikara,

especially Brihmana variety of basti like

Mamsa rasa Basti and yapana basti

(contains madhanaphala) with kala and

karma format, considering the condition,

can be administered44. Tikta Ghruthas,

Ashwanganadha ghrutha and Chagalayadi

ghrutha can be administered as Anuvanasa

basti45-46. Virechana and basti are

explained in the principle treatment of

Medomamsa dusti47. Nasya has less

importance ,it is assumed that it can be used

for the treatment of various associated

symptoms like depression due to its mana

prasdana action 48 .After the purification

Rasyana therapy can be adopted. Charaka

and Yogaratnakar told in the treatment

principle of Vataroga that upakarmas like

Abhyanga, Svedana are having prime

treatment modalities 49. Both types of

Snehana, bahya and abhyantra helps to

pacifies the vata dosha50 . Abhyanga a

variety of bhaya sneha with oil like Bala –

ashwagandha-lakshadi taila,

Mahanaryana Taila and Mahamamsadi

taila are vataghana and improves the

tonicity of the muscle51-52. Whereas

swedana like Shastikashaali pinda swedana

also improves the tone of the body 53.

Swedana karma increases the metabolic

activity which in turn increases the oxygen

demand and blood flow.

Udvartana- Due to Medo-Kaph har

property it is very effective to treat

hypertrophy of calf muscles and strength of

muscles [54] It also stimulates nerve ending,

relax muscles and relives pain 55 .

Abhyanga - It stimulates circulatory

system, vasodilatation resulting to

nourishment & strengthening of muscles,

reduces connective tissue thickening and

provide flexibility by decreasing fibrous

adhesions from hypertrophied muscles. It

has shown reduction in toe walking,

relieving contractures, nourishment of

atrophied muscles, increasing muscle

power and assisting muscle tone56.

Shashtika Shali Pinda Sweda

1. It should be applied followed by

Abhyanga; muscular dystrophy is the result

of Medo-Mamsa imbalance disorders that’s

why SSPS was found very effective for

nourishment as well as providing strength

of the muscles and nervous tissue to the

affected child.

2. Brimhaniya Snehika sudation done by

bolus of luke warm Shashtika Shali (Oryza

Sativa Linn) with Vatahara Kwatha and

milk. Balamoola Kwatha as Vatahara

Kwatha for SSPS was used due to its

Kapha-Pitta-Vatahara properties, 57 and

Balamool kwatha was its ability to alleviate

Saptadhatugata-vata (normalize the

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functions of Vayu in all the tissues of body,

due to its main ingredient is Bala which is

best drug in alleviating Vata dosha 58 .

Swedana - Fomentation has been

demonstrated to produce decrease in

gamma activity, which reduces stretch on

muscle spindles resulting in decreased

muscle spasm. Elevating muscle

temperature can also alter strength and

endurance. It also results in decreased joint

stiffness and increased tissue extensibility,

thus facilitating ease of motion and range of

movements59. Due to Swedana

vasodilatation occur which stimulates the

superficial nerve endings which leads to

reflex stimulation of sweat glands in the

areas exposed to heat. This rise in

temperature induces muscle relaxation and

increases the efficacy of muscle action as

the increased blood supply ensures the

optimum condition for the muscle

contraction 60 . The secretion of sweat is

under nervous system control. The hair of

the skin is tactile sense organs and their

secretion produces some nervous changes

and can bring about changes in conduction

of nerve stimuli, by changing sodium-ion

concentration.

Virechana - Samyaka Virechana do

Srotovishuddhi, Agni Vriddhi and Vata

Anulomana. Which leads to absorption of

bruhmana & rasayana dravya61.

Basti- It is to be instilled as karma / kaala

basti, considering it as Gambheer

dhatugata vikara62.

Yapana basti -acts as lekhana &

brumhana. It is medohara, increases agni

63. It has regulating effect on gut- brain

(ENS)64 .

Anuvasanabasti –

Anuvasana Basti Krama is the treatment

for Vata Vyadhies (diseases occurring due

to vitiation of Vata), Pakwashaya (Vata

Sthana), which is the main site for action of

Vasti dravya, when the Vata is controlled

then pitta and kapha function in body also

corrected, due to which agni got corrected

and proper Mamsa and Meda dhatu

(muscles tissue )formation occur. Some

examples of Basti which can be used in

muscular dystrophy i.e Mamsa rasa basti

and Mustadiyapana basti, improves

dhatukshaya (depletion of body tissue) 65 .

Nasya – has a property of Mana prasadana

action 66.

CONCLUSION

In Modern medicine there is no specified

treatment for Duchene Muscular

dystrophy.In Ayurveda Purva-

Panchakarma procedures (Snehana, SSPS),

Basti is useful in the long term management

of DMD. Basti controls the Vata and

improves Agni, balancing Doshas,

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eliminate metabolic toxins from

Dhatu.Various research works has been

done on Vatavyadhi in various institute of

India, where it can be concluded that herbo-

mineral medicine along with Panchakarma

therapy has a major role to prevent further

complication of DMD.In Ayurveda,

panchakarma procedures can provide

patients of DMD, longer survival.

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